Cardiac Transplantation for Refractory Catecholaminergic Polymorphic Ventricular Tachycardia
Sumon Roy1, Winston Hong2, Melissa Wasilewski2
1Department of Cardiovascular Disease, Virginia Commonwealth University Health System, Richmond, Virginia.
JACC. Case Reports
|July 28, 2021
Summary
Catecholaminergic polymorphic ventricular tachycardia (CPVT) can be refractory to standard treatments. Cardiac transplantation offers a curative option for patients with severe, drug-resistant CPVT and recurrent ventricular tachycardia.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare, inherited arrhythmia syndrome.
- Patients experience life-threatening ventricular arrhythmias triggered by adrenergic stress.
- Standard treatments include beta-blockers, antiarrhythmic drugs, and left cardiac sympathetic denervation.
Observation:
- A patient with CPVT presented with syncope and recurrent ventricular tachycardia lasting 11 minutes.
- The patient's condition was refractory to maximal antiarrhythmic drug therapy and bilateral sympathetic denervation.
- Twenty-one electrical shocks were administered without sustained arrhythmia termination.
Findings:
- Cardiac transplantation was performed as a curative treatment for the refractory ventricular arrhythmias.
- This case highlights the potential of heart transplantation in managing severe CPVT.
Implications:
- Cardiac transplantation may be a viable therapeutic option for select CPVT patients with intractable arrhythmias.
- Further research is needed to establish the long-term efficacy and safety of transplantation in CPVT.
- This case underscores the importance of considering advanced treatment modalities for complex inherited arrhythmia syndromes.
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